title: Ventricular fibrillation in a patient with Pompe disease: a cautionary tale.
abstract: Pompe disease is a rare genetic disorder resulting from a deficiency of the acid α-glucosidase enzyme. Although arrhythmias occur in these patients undergoing general anesthesia, they have not received sufficient emphasis in pediatric cardiology. We report a case of an infant with Pompe disease who experienced ventricular fibrillation during induction of anesthesia.
authors.affiliation: Department of Pediatrics, Tulane University, New Orleans, LA, USA.
authors.forename: Holly C
authors.initials: HC
authors.lastname: Desena
authors.forename: Michael R
authors.initials: MR
authors.lastname: Brumund
authors.forename: Duane
authors.initials: D
authors.lastname: Superneau
authors.forename: Christopher S
authors.initials: CS
authors.lastname: Snyder
mesh_terms: D000768:Anesthesia, General / Q000009:adverse effects*; D006332:Cardiomegaly / Q000209:etiology; D002405:Catheterization, Central Venous; D004562:Electrocardiography; D056947:Enzyme Replacement Therapy; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007223:Infant; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy